Alpha1-antitrypsin deficiency: the radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: a survey by the British thoracic association*
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Thoracic CT Imaging in AATD
2017, Alpha-1-antitrypsin Deficiency: Biology, Diagnosis, Clinical Significance, and Emerging TherapiesAlpha-1-Antitrypsin Deficiency: Epidemiological Studies and Other AATD Associated Diseases
2017, Alpha-1-antitrypsin Deficiency: Biology, Diagnosis, Clinical Significance, and Emerging TherapiesAlpha-1 antitrypsin deficiency
2011, Respiratory Medicine CMECitation Excerpt :In the lung, emphysema is the most common manifestation. The emphysema associated with AAT deficiency tends to be early in onset (i.e., in the fourth and fifth decades), panacinar in pathology and disproportionate in its effect on the lung bases (compared to the more apical distribution in AAT replete patients).6–8 Evidence for the association of bronchiectasis with AAT deficiency is mixed.
Alpha-1 antitrypsin deficiency
2010, Respiratory MedicineCitation Excerpt :In the lung, emphysema is the most common manifestation. The emphysema associated with AAT deficiency tends to be early in onset (i.e., in the fourth and fifth decades), panacinar in pathology and disproportionate in its effect on the lung bases (compared to the more apical distribution in AAT replete patients).6–8 Evidence for the association of bronchiectasis with AAT deficiency is mixed.
Lung Volume Reduction Surgery in Patients With Emphysema and α-1 Antitrypsin Deficiency
2007, Annals of Thoracic SurgeryCitation Excerpt :For example, in contrast to the roughly even gender distribution of subjects in the National Heart, Lung, and Blood Institute AAT Registry [11], 80% of AAT-deficient NETT participants were male. Also, although the typical panlobular emphysema of AAT deficiency more commonly involves the lung bases [12–15] in NETT, 70% of AAT-deficient subjects undergoing LVRS had upper-lobe-predominant emphysema. A recent detailed CT analysis of PI*ZZ subjects by Parr and colleagues [13], for example, indicated that 64% demonstrated basal predominance of emphysema and that only 36% had upper-lobe-predominant emphysema.
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This study was coordinated by Dr D. C. S Hutchison on behalf of a subcommittee of the Research Committee of the British Thoracic Association. Members of the subcommittee were: Professor J. G. Scadding (Chairman), Mr G. Berry, Dr P. J. L. Cook, Professor C. M. Fletcher (resigned 1979), Dr E. A. Hills (resigned 1980), Dr D. C. S. Hutchison, Dr S. J. Pearce, Dr G. M. Sterling and Professor M. Turner-Warwick (resigned 1975).